Zusammenfassung
Die neuen Behandlungsmöglichkeiten für Nierenzellkarzinome haben das Interesse an der Histogenese und den molekularen Veränderungen von Nierentumoren erheblich verstärkt. Die aktuelle Klassifikation der Nierentumoren beruht auf spezifischen molekularen Veränderungen und charakteristischen biologischen Eigenschaften. Es wurden in den letzten Jahren mehrere neue Tumortypen identifiziert, deren Kenntnis erheblichen Einfluss auf die Auswahl der Behandlungsstrategien hat. In der vorliegenden Übersichtsarbeit wird die aktuelle Nomenklatur der Nierenneoplasien unter besonderer Berücksichtigung neuer Nierentumortypen diskutiert. Die Kenntnis von Xp11-Translokationskarzinomen und Nierentumoren mit dominanter Zystenbildung ist diagnostisch relevant. Bestimmte Genotyp-Phänotyp-Korrelationen haben zunehmenden Einfluss auf die Auswahl der Therapieverfahren und die Einschätzung der Prognose. Gleichzeitig führt eine umfangreichere pathologisch-anatomische Aufarbeitung von Nephrektomiepräparaten unter besonderer Berücksichtigung des Nierenhilus zu einer korrekteren TNM-Stadienklassifikation mit genauerer Prognosevorhersage mit sich.
Abstract
New treatment options for renal cell cancer have increased the interest in the histogenesis and in molecular alterations of renal tumors. The current WHO classification of renal cancer is based on specific molecular alterations and characteristic biologic behavior. During recent years novel tumor types have been identified with immediate impact for the selection of new treatment modalities. In this review the current classification of renal cancer with special emphasis on novel renal tumor types will be discussed. Knowledge of Xp11 translocation carcinomas and renal tumors with characteristic cyst formation is diagnostically relevant. In addition this review focuses on reassessing the current TNM staging system for renal cell cancer. Retrospective studies have shown the relevance of renal sinus invasion for stage evaluation.
Literatur
McLaughlin JK, Lipworth L (2000) Epidemiologic aspects of renal cell cancer. Semin Oncol 27(2):115–123
Ceschi M, Gutzwiller F, Moch H et al (2007) Epidemiology and pathophysiology of obesity as cause of cancer. Swiss Med Wkly 137(3–4):50–56
Thoenes W, Stoerkel S, Rumpelt H (1986) Histopathology and classification of renal cell tumors (adenomas, oncocytomas, and carcinomas): the basic cytological and histopathological elements and their use for diagnostics. Pathol Res Pract 181:125–143
Eble J, Sauter G, Epstein J, Sesterhenn I (2004) Tumours of the Kidney. In: Tumours of the Urinary System and Male Genital Organs. IARC, Lyon
Gnarra JR, Tory K, Weng Y et al (1994) Mutations of the VHL tumour suppressor gene in renal carcinoma. Nat Genet 7(1):85–90
Kovacs G, Fuzesi L, Emanual A, Kung HF (1991) Cytogenetics of papillary renal cell tumors. Genes Chromosom Cancer 3(4):249–255
Amin MB, Paner GP, Alvarado-Cabrero I et al (2008) Chromophobe renal cell carcinoma: histomorphologic characteristics and evaluation of conventional pathologic prognostic parameters in 145 cases. Am J Surg Pathol 32(12):1822–1834
Speicher M, Schoell B, Du Manoir S et al (1994) Specific loss of chromosomes 1, 2, 6, 10, 13, 17, and 21 in chromophobe renal cell carcinomas revealed by comparative genomic hybridization. Am J Pathol 145(2):356–364
Moch H, Gasser T, Amin MB et al (2000) Prognostic utility of the recently recommended histologic classification and revised TNM staging system of renal cell carcinoma: a Swiss experience with 588 tumors. Cancer 89(3):604–614
Eichelberg C, Junker K, Ljungberg B, Moch H (2009) Diagnostic and prognostic molecular markers for renal cell carcinoma: a critical appraisal of the current state of research and clinical applicability. Eur Urol 55(4):851–863
Amin MB, MacLennan GT, Gupta R et al (2009) Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. Am J Surg Pathol 33(3):384–392
Tickoo SK, Deperalta-Venturina MN, Harik LR et al (2006) Spectrum of epithelial neoplasms in end-stage renal disease: An experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. Am J Surg Pathol 30(2):141–153
Rakozy C, Schmahl GE, Bogner S, Storkel S (2002) Low-grade tubular-mucinous renal neoplasms: morphologic, immunohistochemical, and genetic features. Mod Pathol 15(11):1162–1171
Parham DM, Roloson GJ, Feely M et al (2001) Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms‘ Tumor Study Group Pathology Center. Am J Surg Pathol 25(2):133–146
Casella R, Moch H, Rochlitz C et al (2001) Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol 39:613–617
Argani P, Faria PA, Epstein JI et al (2000) Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol 24(8):1087–1096
Moch H, Wodzynski A, Guillou L, Nickeleit V (2003) Primary renal synovial sarcoma. A new entity in the morphological spectrum of spindle cell renal tumors. Pathologe 24(6):466–472
Argani P, Antonescu CR, Illei PB et al (2001) Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am J Pathol 159(1):179–192
Argani P, Antonescu CR, Couturier J et al (2002) PRCC-TFE3 renal carcinomas: morphologic, immunohistochemical, ultrastructural, and molecular analysis of an entity associated with the t(X;1)(p11.2;q21). Am J Surg Pathol 26(12):1553–1566
Argani P, Lae M, Hutchinson B et al (2005) Renal Carcinomas with the t(6;11)(p21;q12): Clinicopathologic features and demonstration of the specific alpha-TFEB gene fusion by immunohistochemistry, RT-PCR, and DNA PCR. Am J Surg Pathol 29(2):230–240
Bruder E, Passera O, Harms D et al (2004) Morphologic and molecular characterization of renal cell carcinoma in children and young adults. Am J Surg Pathol 28(9):1117–1132
Argani P, Lal P, Hutchinson B et al (2003) Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am J Surg Pathol 27(6):750–761
Argani P, Olgac S, Tickoo SK et al (2007) Xp11 Translocation renal cell carcinoma in adults: Expanded clinical, pathologic, and genetic spectrum. Am J Surg Pathol 31(8):1149–1160
Adsay NV, Eble JN, Srigley JR et al (2000) Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol 24(7):958–970
Moch H, Schurch LV, Sulser T, Terracciano L (2004) Mixed epithelial and stromal tumor of the kidney. Pathologe 25(5):356–361
Staller P, Sulitkova J, Lisztwan J et al (2003) Chemokine receptor CXCR4 downregulated by von Hippel-Lindau tumour suppressor pVHL. Nature 425(6955):307–311
Frew IJ, Thoma CR, Georgiev S et al (2008) pVHL and PTEN tumour suppressor proteins cooperatively suppress kidney cyst formation. Embo J 27(12):1747–1757
Thoma CR, Frew IJ, Hoerner CR et al (2007) pVHL and GSK3beta are components of a primary cilium-maintenance signalling network. Nat Cell Biol 9(5):588–595
Schraml P, Frew IJ, Thoma CR et al (2009) Sporadic clear cell renal cell carcinoma but not the papillary type is characterized by severely reduced frequency of primary cilia. Mod Pathol 22(1):31–36
Thoma CR, Frew IJ, Krek W (2007) The VHL tumor suppressor: riding tandem with GSK3beta in primary cilium maintenance. Cell Cycle 6(15):1809–1813
Peralta-Venturina M de, Moch H, Amin M et al (2001) Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases. Am J Surg Pathol 25(3):275–284
Moch H, Artibani W, Delahunt B et al (2009) Reassessing the current UICC/AJCC TNM staging for renal cell carcinoma. Eur Urol 56:636–643
Bonsib SM (2005) T2 clear cell renal cell carcinoma is a rare entity: a study of 120 clear cell renal cell carcinomas. J Urol 174(4 Pt 1):1199–1202; discussion 202
Bonsib SM (2006) Renal lymphatics, and lymphatic involvement in sinus vein invasive (pT3b) clear cell renal cell carcinoma: a study of 40 cases. Mod Pathol 19(5):746–753
Bonsib SM (2007) Renal veins and venous extension in clear cell renal cell carcinoma. Mod Pathol 20(1):44–53
Interessenkonflikt
Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Moch, H. Neue Aspekte der Pathologie des Nierenzellkarzinoms. Onkologe 16, 131–139 (2010). https://doi.org/10.1007/s00761-009-1755-4
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00761-009-1755-4